Treatment

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How might nevoid basal cell carcinoma syndrome be treated?

The features of nevoid basal cell carcinoma syndrome (NBCCS) should be evaluated and treated by specialists who are experienced with the condition (such as oral surgeons, dermatologists, plastic surgeons, and medical geneticists).

If a medulloblastoma is detected early enough, it may be treated by surgery and chemotherapy.[1] Jaw keratocysts usually need to be surgically removed. Early treatment of basal cell carcinomas is necessary to prevent long-term cosmetic problems, particularly on the face. Surgical removal is often supplemented by other treatments such as cryotherapy, laser treatment, and/or photodynamic therapy.[2] Radiation therapy is not recommended because it can provoke the development of more tumors.[1] Some people may need long term treatment with oral retinoids such as isotretinoin or acitretin.

Cardiac fibromas may not cause symptoms, but they should be monitored by a cardiologist. If ovarian fibromas need surgical treatment, it is typically recommended that ovarian tissue is preserved even though it involves a risk of recurrence.[2]

Management Guidelines

GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.

Clinical Trials & Research for this Disease

ClinicalTrials.gov lists trials that are studying or have studied Nevoid basal cell carcinoma syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. There is a study titled Evaluation and Treatment of Patients with Dermatologic Diseases which may be of interest to you. To find this trial, click on the link above.

Orphanet lists clinical trials, research studies, and patient registries enrolling people with this condition. Click on Orphanet to view the list.

The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. There is a study titled Dermatology Consultation Clinic and Clinical Research that may be of interest to you. You may want to contact the investigator, Maria Turner (maria.turner@nih.gov) to learn more.